超越腺体:在Sjögren综合征的神经学表现的深入视角。

Alexandria Voigt, Sukesh Sukumaran, Cuong Q Nguyen
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引用次数: 8

摘要

原发性Sjögren综合征(pSjS)是一种自身免疫性疾病,其特征是干眼(干眼或口干)症状、抗ss - a (Ro)或抗ss - b (La)自身抗体和外分泌腺淋巴细胞浸润。据估计,美国有40万至310万人患有SjS,占总人口的0.1-3%,其中女性患SjS的可能性是男性的9倍。频率持续上升,并伴有多因素病因,使其成为一种具有挑战性的疾病,以管理和治疗。由于缺乏依赖人工唾液和眼润滑剂或免疫抑制剂等替代疗法的治疗方法,这种疾病的治疗仍然存在问题。为了进一步使疾病的管理复杂化,有许多多系统表现,特别是周围神经病变与疾病发作的后期相关。越来越多的证据表明中枢神经系统参与其中。目前尚不清楚与SjS相关的免疫反应失调和神经病变的潜在原因和影响。在这篇综述中,我们对psj中记录的关键神经功能障碍进行了深入研究。具体来说,我们讨论了患病率、症状和目前的治疗方法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Beyond the Glands: An in-Depth Perspective of Neurological Manifestations in Sjögren's Syndrome.

Primary Sjögren's Syndrome (pSjS) is an autoimmune disease characterized by sicca (xerophthalmia or xerostomia) symptoms, anti-SS-A (Ro) or anti-SS-B (La) autoantibodies, and lymphocytic infiltrates in the exocrine glands. Disease incidence is estimated to be 0.1-3% of the general population with 0.4-3.1 million individuals in the US with women being nine times more likely to be afflicted with SjS than men. The frequency continues to rise accompanied with the multi-factorial etiology making it a challenging disease to manage and treat. Treatment of this disease remains problematic due to the lack of therapeutic treatments relying on replacement therapies such as artificial saliva and eye lubricants or immunosuppressive agents. To further complicate the management of the disease, there are number of multi-systemic manifestations specifically peripheral neuropathy associated with later stage of disease onset. Increasingly, there is mounting evidence that suggests the involvement of central nervous system. It remains to be determined the underlying cause and effect of the dysregulated immune response and the neuropathy associated with SjS. In this review, we provided an in-depth look at key neurological dysfunctions documented to occur in pSjS. Specifically, we discussed the prevalence, symptomology, and current treatments.

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